Malignant presacral ghrelinoma with long-standing hyperghrelinaemia
(2015) In Uppsala Journal of Medical Sciences 120(4). p.299-304- Abstract
- Background. A 57-year old man with low-back pain was found to have a 3 x 3 x 3 cm presacral neuroendocrine tumour (NET) with widespread metastases, mainly to the skeleton. His neoplastic disease responded well to peptide receptor radionuclide therapy (PRRT) with the radiotagged somatostatin agonist Lu-177-DOTATATE. During almost 10 years he was fit for a normal life. He succumbed to an intraspinal dissemination. Procedures. A resection of the rectum, with a non-radical excision of the adjacent NET, was made. In addition to computerized tomography (CT), receptor positron emission tomography (PET) with Ga-68-labelled somatostatin analogues was used. Observations. The NET showed the growth pattern and immunoprofile of a G2 carcinoid. A... (More)
- Background. A 57-year old man with low-back pain was found to have a 3 x 3 x 3 cm presacral neuroendocrine tumour (NET) with widespread metastases, mainly to the skeleton. His neoplastic disease responded well to peptide receptor radionuclide therapy (PRRT) with the radiotagged somatostatin agonist Lu-177-DOTATATE. During almost 10 years he was fit for a normal life. He succumbed to an intraspinal dissemination. Procedures. A resection of the rectum, with a non-radical excision of the adjacent NET, was made. In addition to computerized tomography (CT), receptor positron emission tomography (PET) with Ga-68-labelled somatostatin analogues was used. Observations. The NET showed the growth pattern and immunoprofile of a G2 carcinoid. A majority cell population displayed immunoreactivity to ghrelin, exceptionally with co-immunoreactivity to motilin. Somatostatin receptor scintigraphy and Ga-68-DOTATATE PET-CT demonstrated uptake in the metastatic lesions. High serum concentrations of total (desacyl-)ghrelin were found with fluctuations reflecting the severity of the symptoms. In contrast, the concentrations of active (acyl-)ghrelin were consistently low, as were those of chromogranin A (CgA).Conclusions. Neoplastically transformed ghrelin cells can release large amounts of desacyl-ghrelin, evoking an array of non-specific clinical symptoms. Despite an early dissemination to the skeleton, a ghrelinoma can be compatible with longevity after adequate radiotherapy. (Less)
Please use this url to cite or link to this publication:
https://lup.lub.lu.se/record/8525913
- author
- Falkmer, Ursula G. ; Gustafsson, Thomas ; Wenzel, Ralf ; Wierup, Nils ; Sundler, Frank LU ; Kulkarni, Harshad ; Baum, Richard P. and Falkmer, Sture E.
- organization
- publishing date
- 2015
- type
- Contribution to journal
- publication status
- published
- subject
- keywords
- Desacyl, acyl-ghrelin, ghrelinoma, hyperghrelinaemia, Lu-177 therapy, motilin, presacral carcinoid, skeletal neuroendocrine tumour, dissemination, theranostics
- in
- Uppsala Journal of Medical Sciences
- volume
- 120
- issue
- 4
- pages
- 299 - 304
- publisher
- Taylor & Francis
- external identifiers
-
- wos:000365684900010
- scopus:84945138508
- pmid:26095011
- ISSN
- 0300-9734
- DOI
- 10.3109/03009734.2015.1054453
- language
- English
- LU publication?
- yes
- id
- 2e675ab2-8453-457d-8613-d6439739f12e (old id 8525913)
- date added to LUP
- 2016-04-01 10:10:30
- date last changed
- 2022-03-12 02:55:35
@article{2e675ab2-8453-457d-8613-d6439739f12e,
abstract = {{Background. A 57-year old man with low-back pain was found to have a 3 x 3 x 3 cm presacral neuroendocrine tumour (NET) with widespread metastases, mainly to the skeleton. His neoplastic disease responded well to peptide receptor radionuclide therapy (PRRT) with the radiotagged somatostatin agonist Lu-177-DOTATATE. During almost 10 years he was fit for a normal life. He succumbed to an intraspinal dissemination. Procedures. A resection of the rectum, with a non-radical excision of the adjacent NET, was made. In addition to computerized tomography (CT), receptor positron emission tomography (PET) with Ga-68-labelled somatostatin analogues was used. Observations. The NET showed the growth pattern and immunoprofile of a G2 carcinoid. A majority cell population displayed immunoreactivity to ghrelin, exceptionally with co-immunoreactivity to motilin. Somatostatin receptor scintigraphy and Ga-68-DOTATATE PET-CT demonstrated uptake in the metastatic lesions. High serum concentrations of total (desacyl-)ghrelin were found with fluctuations reflecting the severity of the symptoms. In contrast, the concentrations of active (acyl-)ghrelin were consistently low, as were those of chromogranin A (CgA).Conclusions. Neoplastically transformed ghrelin cells can release large amounts of desacyl-ghrelin, evoking an array of non-specific clinical symptoms. Despite an early dissemination to the skeleton, a ghrelinoma can be compatible with longevity after adequate radiotherapy.}},
author = {{Falkmer, Ursula G. and Gustafsson, Thomas and Wenzel, Ralf and Wierup, Nils and Sundler, Frank and Kulkarni, Harshad and Baum, Richard P. and Falkmer, Sture E.}},
issn = {{0300-9734}},
keywords = {{Desacyl; acyl-ghrelin; ghrelinoma; hyperghrelinaemia; Lu-177 therapy; motilin; presacral carcinoid; skeletal neuroendocrine tumour; dissemination; theranostics}},
language = {{eng}},
number = {{4}},
pages = {{299--304}},
publisher = {{Taylor & Francis}},
series = {{Uppsala Journal of Medical Sciences}},
title = {{Malignant presacral ghrelinoma with long-standing hyperghrelinaemia}},
url = {{http://dx.doi.org/10.3109/03009734.2015.1054453}},
doi = {{10.3109/03009734.2015.1054453}},
volume = {{120}},
year = {{2015}},
}